Common Myths and Misconceptions in Pediatric Neurology Common Myths and Misconceptions in Pediatric

Common Myths and Misconceptions in Pediatric Neurology

Michael Zimbric, MD UCSD/Rady Children’s Hospital-San Diego Department of Neurology

2

Disclosures

• None

Objectives

• To outline 4 common complaints or reasons for referral to Neurology, common misconceptions associated with them, and how (most) child neurologists see these issues.

• To introduce or reintroduce clinical pathways for headache and other complaints.

Misconception #1

Kids with headaches might have a brain tumor

Two types of headaches

Primary Headache

• Tension type HA

• Migraine

• TAC’s

• Cluster HA

• SUNCT

• “Other” primary headaches

• NDPH

• Idiopathic stabbing HA

• Hemicrania continua…

Secondary Headache

• Tumor/Mass

• Vascular lesion

• Increased ICP due to other causes

• Post-ictal

• Paranasal sinus disease

• Other illnesses

Characteristics of secondary HA

Also known as “red flags”

• Any new/unexplained neurological exam abnormality

• Constant, slowly increasing headache

• Neurocutaneous syndrome

• Age less than 3 years (+/-)

• +/- Sudden onset of headache

• Acute “worst headache of life”

• +/- Headache with exertion

• +/- Headache on waking in morning or during night

• +/- Posterior location

• ++/- Presence of VP shunt

Time Course:

Chronic, progressive

• Most ominous, can imply increased intracranial pressure

• Tumor, hydrocephalus, IIH, chronic meningitis, abscess, SDH

Chronic, non progressive

• Over 4 months, >15 HA days/month, >4hrs/day

Mixed

• Usually Acute, recurrent on top of chronic daily/ non progressive HA.

Acute

• In otherwise healthy child, usually due to viral illness

• With focal neurological signs, can be ICH

• Severe, with fever can be due to meningitis

Acute, recurrent

• Attacks of headache separated by symptom-free intervals.

• Migraine, Tension Type Headache

• Partial seizures, substance abuse, Cluster HA, recurrent Trauma

Brain Tumor Headache

Chronic and progressive pattern

AM or nocturnal onset/occurrence

Pernicious vomiting, especially in morning

Personality change

Declining school performance

Diplopia

Head tilt

Gait changes

Exam Findings to check BP measurement

Sinus tenderness

TMJ clicks or pain

Neck range of motion, shoulder muscle tenderness

Mental status exam suggesting depression

OFC measurement

Optic disks

Eye movements

Pronator drift

Ataxia

Abnormal DTR’s

When to Image? • From --AAN Guideline on Evaluation of Headache in Children and

Adolescents: http://www.neurology.org/content/59/4/490.full#ref-1

• Data on 605 children out of 1275 who had: Undergone neuroimaging

• Been examined by a neurologist

Found:

14 (2.3%) with nervous system lesions that required surgical treatment.

All 14 of those children had definite abnormalities on exam.

No patient with a normal examination had a lesion that required surgical treatment.

Misconception #1 Summary:

- Patients presenting with recurrent, isolated headaches who have a normal neurological exam and no red flags have an extremely low chance of having a brain tumor

- The time course is important

- The exam is important

Misconception #2 Febrile seizures in an otherwise normal child could indicate epilepsy and require an EEG.

Types of Febrile Seizures

Simple Febrile Convulsions

Generalized/ non focal

Less than 15 minutes

Most are much shorter

Once in a 24 hour period

In any of these, the fever may come well after the seizure!

Complex Febrile Seizures

Focal

Over 15 minutes

Multiple within 24 hours

Febrile Status Epilepticus (FSE)

Over 30 minutes

Simple Febrile Seizures Risk of future epilepsy is between 2-3%

That of the general pediatric population is about 1-2%

EEG is not shown to be helpful in identifying who is at higher risk for febrile seizure recurrence or future epilepsy*.

*Febrile Seizures: Guideline for the Neurodiagnostic Evaluation of the Child With a Simple Febrile Seizure: http://pediatrics.aappublications.org/content/127/2/389.long

**Kuturec M et al. Febrile seizures: is the EEG a useful predictor of recurrences? Clin Pediatr (Phila). 1997;36(1):31–36

Kuturec et al found that simple febrile seizure recurrence rate was not significantly different between patients with normal or abnormal EEGs**.

Complex Febrile Seizures More disagreement within the literature, among neurologists.

There is a higher risk for epilepsy among these patients:

Annegers et al* studied 687 children with FS into adulthood; 3 risk factors for developing epilepsy were identified: focal FS, prolonged FS, repeated within 24 hrs. Risk of developing epilepsy was 2.4%, 6-8%, 17- 22%, and 49% among patients with zero, 1, 2, or 3 of those, respectively

Natn’l Collaborative Perinatal Project** found children with abnormal neurological development and whose first FS was complex (for any reason) has 9.2% chance of afebrile seizures by 7 years of age, 18x higher than children w/o h/o FS. *Annegers et al. Factors prognostic of unprovoked seizures after febrile convulsions. N Eng J Med. 1987 Feb 26;316(9): 493-8.

**Nelson KB, Ellenberg JH. Predisposing and causative factors in childhood epilepsy. Epilepsia. 1987;28 Suppl 1: S16-24.

Complex Febrile Seizures While it is tempting to recommend more testing (EEG) in patients with complex febrile seizures, the small amount of literature has not supported that it is helpful:

Maytal et al found on retrospective review of 33 patients with CFS that the rate of EEG abnormality is low and similar to the reported rate of EEG abnormality in children with simple febrile seizures (about 8.6%)*.

No study has been able to find any significant differences between EEG abnormalities seen in patients with simple vs complex febrile seizures.

No study has been able to show that EEG abnormalities in patients with CFS reliably predicts epilepsy.

*Maytal et al. The Value of Early Postictal EEG in Children with Complex Febrile Seizures. Epilepsia. 2000 Feb;41(2):219-21

Complex Febrile Seizures Conclusion: We do not recommend EEG for patients with complex febrile seizures.

If an EEG is completed however, we might consider altering management if one or more of the following is present:

a. Generalized epileptiform discharges

b. Focal slowing

c. Very frequent or abundant focal epileptiform discharges

HOWEVER, the EEG should never be used in place of a good developmental history and neurological exam. In other words, abnormalities on either of those is a much better guide of diagnostic workup than an EEG abnormality

Misconception #2 Summary:

- The risk of epilepsy among otherwise normal patients with febrile seizures is low

- Complex febrile seizures carry a higher (though still usually low) risk of future epilepsy.

- EEG is not recommended for patients with febrile seizures

- A good developmental history and examination is important.

Misconception #3

Tics always need to be treated (with medication)

Tics

Definition:

A repeated movement or noise that is typically done frequently, is suppressible, and stereotyped. It is non-rhythmical and discrete. There is a pre-monitory urge. The movement or noise is involuntary or semi-voluntary.

Tics Motor: Eye blinking, facial grimacing, nose wrinkling, eyebrow raising, eye rolling, shoulder shrugs, head shakes or quick jerks, abdominal movements, wrist or ankle movements…

Transient motor tics occur in 10-25% of all children

Vocal: Sniffs, coughs, hums, groans, screeches, gasps, clicks, bilabial trills, screams, words or brief phrases…

Rarely coprolalia

Misconception #3 and 1/2 Having both motor and vocal tics means you have Tourette syndrome

(and must be treated with medication)

Tourette Syndrome Definition:

DSM-V Diagnostic Criteria • Onset before 18 years of Age • Multiple Motor and 1 or More Vocal Tics (do not need to be present

concurrently) • Tics not secondary to substance or other medical condition • Duration > 1year (may wax and wane in frequency)

TOURETTE = (motor + vocal) x 1 year

Tourette Syndrome occurs in 1-4% of mainstream children with a 25% incidence in children with special needs

4:1 male to female

When to medicate? IF

• Experiencin